Signaling pathway deregulation and molecular alterations across pediatric medulloblastomas.

Fiche publication


Date publication

mai 2018

Journal

Neuro-Chirurgie

Auteurs

Membres identifiés du Cancéropôle Est :
Pr ENTZ-WERLE Natacha, Dr GUERIN Eric


Tous les auteurs :
Lhermitte B, Blandin AF, Coca A, Guerin E, Durand A, Entz-Werlé N

Résumé

Medulloblastomas (MBs) account for 15% of brain tumors in children under the age of 15. To date, the overall 5-year survival rate for all children is only around 60%. Recent advances in cancer genomics have led to a fundamental change in medulloblastoma classification and is evolving along with the genomic discoveries, allowing to regularly reclassify this disease. The previous molecular classification defined 4 groups (WNT-activated MB, SHH-activated MB and the groups 3 and 4 characterized partially by NMYC and MYC driven MBs). This stratification moved forward recently to better define these groups and their correlation to outcome. This new stratification into 7 novel subgroups was helpful to lay foundations and complementary data on the understanding regarding molecular pathways and gene mutations underlying medulloblastoma biology. This review was aimed at answering the recent key questions on MB genomics and go further in the relevance of those genes in MB development as well as in their targeted therapies.

Mots clés

Biology, Genes, Medulloblastomas, Signaling pathway, Update

Référence

Neurochirurgie. 2018 May 15;: